AB0725 MACROPHAGE ACTIVATION SYNDROME IN CHILDREN WITH RHEUMATIC DISEASES: ANALYSIS OF CASE SERIES

Abstract

Background:Macrophage activation syndrome (MAS) is a rare, but severe life-threatening complication of chronic rheumatic disease (RD) in children, which associated with high risks of the multiple organ failure and mortality.Objectives:Tо analyze demographic, clinical and laboratory parameters, timing of MAS and disease outcome in patients (pts) with MAS and RD.Methods:The study included all pts of single center with RD, who developed the MAS. The diagnosis was recognized according to Classification criteria for MAS in sJIA [1].Results:We observed 52 pts with RD and MAS: 31 (59.6%) with sJIA, 19 (36.5%) – SLE, 1(1.9%) – juvenile dermatomyositis (JDM), 1 (1.9%) – overlap syndrome (JIA+JDM). Pts with MAS accounted of 26% of all pts with sJIA, 7.6% of all pts with SLE. The mean age of pts at onset of sJIA was 2.6 y [1.5; 5.75], at onset of SLE – 11.8 y [8.6; 13.95]. The patient with JDM was 6.5 years old, pts with overlap syndrome – 3.5 years old. Male to female ratio was 1:1,7. A total of 63 episodes of MAS was recorded (41 – in sJIA, 20 – SLE, 1 – JDM, 1 - overlap syndrome). 23 pts (44.2%) had MAS at onset, 26 pts (50%) – “classic” MAS (during the course of disease), 3 pts (5.8%) – recurrent MAS. The clinical manifestations of MAS included fever (90.4%), hepatomegaly (50%), pericarditis (46.2%), hemorrhagic rash (32.7%), neurologic involvement (42.3%), lung involvement (34.6%). We found hyperferritinemia in 98%, thrombocytopenia in 78.8%, increased transaminases in 88.5%, hypofibrinogenemia in 40.4%, hypertriglyceridemia in 42.3 % of pts. Most severe course of MAS was established in pts with ferritin levels >1500 ng/ml and with hypertriglyceridemia more than 6.0 mmol/l at an early stage. Bone marrow investigation was performed in 21 pts, and the evidence of haemophagocytosis was confirmed in 8 pts (38%). First features of MAS were fever, sleepiness, lower platelet counts, high TA. Lesions of the skin and mucous were mainly represented by point hemorrhages at an early stage in pts with SLE and MAS, the development of a bright rash with itching was typical for pts with sJIA and MAS. There are no principal differences between course of MAS in sJIA and other RD in children, but mild “subclinical” cases of MAS were observed only in pts with sJIA on biologics. For treatment of MAS all pts were administered high dose of glucocorticoids (per os+iv). Pts with SLE received: cyclophosphamide iv - 5 (26.3%), cyclosporine per os 1 (5.2%), IVIGs - 6 (31.6%), rituximab - 2 pts (10.5%). Pts with sJIA received: cyclosporine per os - 20 (64.5%), IVIGs - 25 (80.6%), 1 etoposide - (3.2%). Patient with overlap syndrome received cyclosporine per os. 8 pts (15.4%) died from MAS (3 with sJIA, 5 – with SLE).Conclusion:MAS can develop in various children’s RD, but more often in pts with sJIA. In our observation more pts had MAS during the course of disease, not at onset. Rapid recognition of MAS can improve treatment outcomes and prognosis.